title: Rehabilitation of children and infants with osteogenesis imperfecta. A program for ambulation.
abstract: Management of children and infants with osteogenesis imperfecta (OI) poses difficult decisions for pediatricians, orthopedists, and physiatrists. These children are frequently frail with disabling bone and joint deformities and fractures. In an eight-year cumulative management of 12 children with OI, a comprehensive program included strengthening exercises to the pelvic girdle and lower extremity muscles, in addition to pool exercises and molded seating to support upright posture. Long leg braces were fitted when the children were able to sit unsupported. All 12 were fitted with braces; nine were functional ambulators, and three were home ambulators. Six children required femoral plating or rodding, two of whom subsequently had the metal removed. Lower extremity fractures averaged one and one-half per year prior to bracing for nine children who had fractures. There was 0.83 fracture per year for the ten children who had fractures after bracing. The degree of femoral bowing increased in four, decreased in four, and remained unchanged in four, while the degree of tibial bowing increased in two, decreased in nine, and remained unchanged in one during the observation period. A comprehensive rehabilitation program and long leg bracing with surgical operations on the femur result in a high level of functional activity for children with OI with an acceptable level of risk for fracture.
authors.affiliation: Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, MD 20892.
authors.forename: L H
authors.initials: LH
authors.lastname: Gerber
authors.forename: H
authors.initials: H
authors.lastname: Binder
authors.forename: J
authors.initials: J
authors.lastname: Weintrob
authors.forename: D K
authors.initials: DK
authors.lastname: Grange
authors.forename: J
authors.initials: J
authors.lastname: Shapiro
authors.forename: W
authors.initials: W
authors.lastname: Fromherz
authors.forename: R
authors.initials: R
authors.lastname: Berry
authors.forename: A
authors.initials: A
authors.lastname: Conway
authors.forename: S
authors.initials: S
authors.lastname: Nason
authors.forename: J
authors.initials: J
authors.lastname: Marini
mesh_terms: D001915:Braces; D002370:Casts, Surgical; D002648:Child; D002675:Child, Preschool; D005081:Exercise Therapy; D005264:Femoral Fractures / Q000453:epidemiology / Q000628:therapy; D005593:Fracture Fixation, Internal / Q000295:instrumentation; D006801:Humans; D015994:Incidence; D007223:Infant; D010013:Osteogenesis Imperfecta / Q000150:complications / Q000503:physiopathology / Q000534:rehabilitation*; D013978:Tibial Fractures / Q000453:epidemiology / Q000628:therapy